Uveitis in pediatric idiopathic orbital inflammatory syndrome

Purpose: To report the clinical presentation, multimodal imaging, and long-term management of two children with idiopathic orbital inflammatory syndrome and associated uveitis. Methods: Observational case series of two pediatric patients with idiopathic orbital inflammatory syndrome and associated uveitis. Results: Both patients presented with severe anterior uveitis with posterior segment manifestations including optic nerve papillitis and macular edema in addition to orbital inflammation with ptosis, eyelid swelling, painful extraocular muscle dysmotility, and optic neuropathy. Due to recurrent orbital and uveitis flares with inability to taper off steroids, both children required systemic immunomodulatory treatment to maintain disease quiescence. Conclusion: Pediatric idiopathic orbital inflammatory syndrome may present with orbital inflammation associated with uveitis requiring systemic immunomodulatory therapy. Orbital inflammation must be considered in the differential diagnosis for pediatric patients presenting with uveitis to determine need for intracranial imaging and systemic treatment. Long-term follow-up is recommended due to risk of recurrence of both orbital and intraocular inflammation.